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Inborn Errors of Metabolism

1. Rationale
Inborn Errors of Metabolism (IEMs) are genetic disorders usually caused by a deficiency of enzymes or its cofactors resulting to the accumulation of toxic metabolites. Nutritional management of IEMs is vital to attain good metabolic control, prevent complications, and ensure proper growth and development.

In the Philippines, there is a limited number of healthcare professionals capable of handling the Nutritional management of IEM as it requires:

  • Deeper knowledge of the genetic disorder and its corresponding Nutritionist restrictions
  • Higher competency on computing  metabolic diet prescription, including the sick-day regimen and the use of the different medical formulas

IEMs started to be detected and diagnosed in 1996 through Newborn Screening (NBS). Six diseases can be screened at the time. In 2012, Expanded Newborn Screening (ENBS) became available, allowing for up to 28 diseases to be detected. With the increase of screened diseases, the number of patients also grew.

With this, additional Nutritionist-Dietitians are needed to handle the Nutritional care of IEM patients. Workshops are necessary to train more Filipino Nutritionist-Dietitians, with the goal of giving them the capacity to provide proper Nutritional care to IEM patients within their reach.
 
2.Objectives
The aim of this training program is to enhance the knowledge and competency of attending clinical Nutritionist-Dietitians from newborn screening continuity clinic hospitals or other regional hospitals and public health Nutritionist-Dietitians, allowing them to manage the Nutritional needs of patients diagnosed with IEMs

At the end of the webinar-workshop, the participants must be able to:

  1. Discuss what is Philippine Expanded Newborn Screening and its role in the prevention of mortality and development of complications IEM
  2. Identify the role of Nutritionist-Dietitians in the system of IEM management in the Philippines (including the referral system, continuity clinics, source and allocation of medical formulas, etc.)
  3. Discuss the basic genetics, pathophysiology, clinical features, and treatment of selected IEMs
  4. Identify and demonstrate the Nutritional care process involved in IEM management for in- and out- patients
  5. Enumerate strategies in conducting dietary counseling among IEM patients.
  6. Discuss IEM dietary management in the global and local setting
3.Target Participants (REGISTRATION IS FULL)
Primary: Dietitians from Newborn Screening Continuity Clinic Hospitals
Secondary: Public Health Dietitians/ Private Hospital Dietitians
 
4.Expected competencies from Clinical Nutritionist-Dietitians and Public Health Dietitians:
Dietitians attending the case study and workshop are expected to perform the following:
  1. Dietary assessment
  2. Interpretation and Computation of IEM diet prescription
  3. Translation of diet prescription to regular food and medical formula
  4. Sick-day regimen computation
  5. Planning of cycle menu for IEM patients
  6. Counseling of IEM patients and caretakers
5.Methodology
A variety of methodologies will be used such as online lectures and presentations, small group (via breakout rooms) and large-group discussions (zoom webinar), computation workshops, case study presentations, online games, etc. Pre-tests, Post-tests and Evaluation surveys will be used as metrics for effectiveness of online learning platform.
 
6.Topics and Speakers
Below is the list of topics and speakers expected to be discussed during the webinar series on Metabolic Dietetics:
 

Suggested Date

Topic

Duration

Contents

Speakers

 

July 28 Tuesday 2:00-4:00PM

National Comprehensive Newborn Screening System/ Role of Dietitians in the NBS Referral System

45 mins
  • What is NBS
  • NBS history
  • ENBS flow of implementation

Maria Melanie Liberty Alcausin, MD

 

August 4 Tuesday 2:00-4:00

Basic Genetics of Inborn Errors of Metabolism*

45 mins
  • Basic terms in Genetics
  • Genetics behind IEM conditions

Mary Ann Abacan, MD

 

 

August 6 Thursday 10:00-12:00

Basic Principles in the Medical Management of Inborn Errors of Metabolism*

45 mins
  • Medical management of common IEMs in the Philippines
  • Challenges and actions

Leniza De Castro- Hamoy, MD

 

August 11 Tuesday 2:00-4:00PM

Developmental Considerations in IEM (Rehab and psychology)*

45 mins
  • Normal feeding-related developmental milestones
  • Developmental outcomes in patients with IEM and implication on feeding

Kaye Napalinga, MD

 

August 14 Wednesday 10:00-12:00

Dietary Management of Amino Acidopathies*

45 mins
  • Background
  • Assessment
  • Diet prescription adjustment and interpretation

Sylvia Estrada,MD

 

August 18 Tuesday 10:00-12:00

Case Discussion/ Computation Workshop on Amino Acidopathies**

1 hour 30 mins
  • Case presentation (newborn, children, maternal, in- patient)
  • Diet planning
  • Counseling
  • Monitoring and Evaluation

Metab Dietitians: Aster Lynn Sur Cecilia Belen Jeanne Ruth Basas Elizabeth Limos

 

August 25 Tuesday 10:00-12:00

Dietary Management of Organic Acidurias*

45 mins
  • Background
  • Assessment
  • Diet prescription adjustment and interpretation

Barbra Cavan, MD

 

August 27 Thursday 10:00-12:00

Case Discussion/ Computation Workshop on Organic Acidurias**

1 hour 30 mins
  • Case presentation
  • Diet planning
  • Counseling
  • Monitoring and Evaluation

Metab Dietitians: Aster Lynn Sur Jessica Yasay Jeanne Ruth Basas Elizabeth Limos

 

September 1 Tuesday 10:00-12:00

Dietary Management of Urea Cycle Defects*

45 mins
  • Background
  • Assessment
  • Diet prescription adjustment and interpretation

Michelle E. Abadingo, MD

 

September 3 Thursday 10:00-12:00

Case Discussion/ Computation Workshop on Urea Cycle Defects**

1 hour 30 mins
  • Case presentation
  • Diet planning
  • Counseling
  • Monitoring and Evaluation

Metab Dietitians: Aster Lynn Sur Jessica Yasay Jeanne Ruth Basas Elizabeth Limos

September 8 Tuesday 2:00-4:00PM

Dietary Management of Fatty Acid Oxidation Disorders*

45 mins
  • Background
  • Assessment
  • Diet prescription adjustment and interpretation

Kristin Gonzales, MD

 
September 10
Thursday
10:00-12:00

Case Discussion/ Computation Workshop on Fatty Acid Oxidation Disorders**

1 hour 30 mins
  • Case presentation
  • Diet planning
  • Counseling
  • Monitoring and Evaluation

Metab Dietitians: Aster Lynn Sur Jessica Yasay Jeanne Ruth Basas Elizabeth Limos
September 15
Tuesday
10:00-12:00
Dietary
Management of
Carbohydrate
Metabolism
Disorders*

45 mins
  • Background
  • Assessment
  • Diet
  • prescription adjustment and interpretation

 

Ebner Bon Maceda, MD

 
September 17
Thursday
10:00-12:00

Case Discussion/ Computation Workshop on Carbohydrate Metabolism Disorders**

1 hour 30 mins
  • Case presentation
  • Diet planning
  • Counseling
  • Monitoring and Evaluation

Metab Dietitians: Aster Lynn Sur Dominga Dawe Jeanne Ruth Basas Elizabeth Limos
September 22
Tuesday
10:00-12:00

IEM Management- Philippine Setting*

45 mins
  • Discussion the use of different standards and tools used in diet planning
  • Available
resources
(books, journals,references)

Elizabeth Limos

 

 

September 24
Thursday
10:00-12:00

Nutrition Care Plan Strategies for IEM*

45 mins
  • Identified Best practices
  • Current Status of Patients
  • Plan of Action

Jeanne Ruth Basas

 
September
29
Tuesday
10:00-12:00
IEM Dietary
Management: Global Perspective*

45 mins
  • International practice on IEM Dietary Management
  • Updates on IEM management
  • International
    Organizations
     
Maureen Evans, PhD
(Royal Children's Hospital)